Jansky-Bielschowsky disease


lipofuscinosis

 [lip″o-fu″sin-o´sis] any disorder due to abnormal storage of lipofuscins.neuronal ceroid-lipofuscinosis any of several genetic lipidoses characterized by progressive neurodegeneration, loss of vision, and a fatal course; included are janský-bielschowsky disease, vogt-spielmeyer disease, and kufs' disease. Formerly known as amaurotic familial idiocy.

Jan·sky-Bi·els·chow·sky dis·ease

(yahn'skē by'els-chov'skē), cerebral sphingolipidosis, early juvenile type.

Jansky-Bielschowsky disease

(jăn′skē-bē′ĕl-shō′skē, -bēl-shôv′-, yän′-)n. A type of neuronal ceroid lipofuscinosis with onset usually between ages two and four.

Bielschowsky,

Max, German neuropathologist, 1869-1940. Bielschowsky-Dollinger syndrome - Synonym(s): Dollinger-Bielschowsky syndromeBielschowsky head tilt testBielschowsky-Jansky disease - Synonym(s): Jansky-Bielschowsky diseaseBielschowsky methodBielschowsky stain - a method of treating tissues with silver nitrate to demonstrate reticular fibers, neurofibrils, axons, and dendrites.Bielschowsky syndrome - Synonym(s): Dollinger-Bielschowsky syndromeDollinger-Bielschowsky syndrome - see under DollingerJansky-Bielschowsky disease - see under Jansky

Jansky,

Jan, Czech physician, 1873-1921. Bielschowsky-Jansky disease - Synonym(s): Jansky-Bielschowsky diseaseJansky classification - the classification of human blood groups now designated O, A, B, and AB.Jansky-Bielschowsky disease - cerebral sphingolipidosis, early juvenile type. Synonym(s): Bielschowsky-Jansky disease